Immunomodulation of TGF-beta1 in mdx mouse inhibits connective tissue proliferation in diaphragm but increases inflammatory response: implications for antifibrotic …

F Andreetta, P Bernasconi, F Baggi, P Ferro… - Journal of …, 2006 - Elsevier
Irreversible connective tissue proliferation in muscle is a pathological hallmark of Duchenne
muscular dystrophy (DMD), a genetic degenerative muscle disease due to lack of the
sarcolemmal protein dystrophin. Focal release of transforming growth factor-beta1 (TGF-β1)
is involved in fibrosis development. Murine muscular dystrophy (mdx) is genetically
homologous to DMD and histopathological alterations comparable to those in DMD muscles
occur in diaphragm of older mdx mice. To investigate the early development of fibrosis and …